Current Status of Difficult Airway Information Dissemination at a Tertiary Pediatric Hospital.

OBJECTIVE: Difficult airway is one of the most common potential risk factors for increased fatality in craniofacial/plastic surgery patients. To date, there is no well-established and all-around difficult airway notification system in China, which has recently been recommended by existing guidelines to improve the care of patients with known difficult airways. This study aimed to investigate the current status of critical information communication concerning difficult airway management in our institute. METHODS: Vital information required to establish a difficult airway registry and notification system was collected through a literature review and expert suggestions. After approval by the Research Ethics Board, 3 online questionnaires were developed and sent to surgeons, anesthesiologists, and proxies of the patients. Knowledge of difficult airways, the willingness to be involved in the difficult airway notification system, and the way to disseminate the information were investigated in the 3 groups. The specific information that was disseminated, required, and available was investigated in the anesthesiologist group. RESULTS: Compared with the surgeons and anesthesiologists, significantly fewer patients knew the definition of a difficult airway and thought it was a potential risk factor. There were no significant differences in the willingness to be informed of the difficulty encountered during airway management. Significant differences were detected in the willingness and way to disseminate the airway information when the participants communicated with different groups. Significantly more patients would disseminate the information to the surgeon and significantly more surgeons would disseminate the information to the patient and the other surgeon. Significantly more anesthesiologists would disseminate the information to the patient and the other anesthesiologists. A significant difference was observed between what was expected and what was available for the anesthesiologist to retrieve the airway information of a patient with known airway difficulty. Significantly more anesthesiologists would notify only the patient of the diagnosis of a difficult airway both oral and written, whereas significantly more anesthesiologists would notify the other anesthesiologist of the specific difficulties in oral only. Most participants agreed to be involved in the difficult airway notification system despite the significantly lower percentage in the patient group (89%). CONCLUSIONS: Difficult airway information dissemination is, at the time of this writing, ineffective, which leads to a large gap between the expectation and practice of the anesthesiologist when caring for a patient with a difficult airway. Thus, a difficult airway registry and notification system should be developed, that has a solid foundation in all the participants.

Incidence of Ventriculomegaly in Patients With Craniosynostosis.

Hydrocephalus is variously associated with syndromic craniosynostosis (CS), while it is randomly encountered in nonsyndromic CS. But actually, the ventriculomegaly in CS is less described. In this study, the authors aim to establish whether ventriculomegaly is common in patients with CS, in both syndromic and nonsyndromic. Retrospective measurements of Evans index (EI) were taken from thin-section computed tomography scans of 169 preoperative CS patients to assess cerebral ventricular volume. EI >0.3 indicates ventricular enlargement. A total of 169 CS patients who underwent computed tomography scan from February 2018 to December 2021 were retrospectively evaluated, including 114 males and 55 females. The average age at diagnosis was 16 months (range: 1-103 mo). Among them, 37 with syndromic CS, including 17 ventricular megaly patients, had an EI >0.3 (46.0%), and 4 of them had intracranial hypertension and needed ventriculoperitoneal shunt treatment before cranial vault remolding. One hundred and thirty-two had nonsyndromic CS (100 single-suture CS, 32 multisuture CS), and 26 of them had an EI of 0.3 or greater (19.7%). Ventrocular megaly is common among patients with CS. Early craniotomy may stabilize ventricular dilation.

Genetic spectrums and clinical profiles of critically ill neonates with congenital auricular deformity in the China Neonatal Genomes Project.

Congenital auricular deformity (CAD) is a complex phenotype that may occur as a single malformation or part of a congenital syndrome. The genetic architecture and utility of next-generation sequencing (NGS) in a sizable cross-sectional study of critically ill neonates with CAD have not yet been systematically investigated. This cross-sectional study investigated the genetic spectrum in critically ill neonates with CADs. Critically ill neonates with CADs (n = 251) were enrolled between August 8, 2016 and October 1, 2022. All neonates underwent NGS. The outcomes were molecular diagnostic yield, spectrum of genetic events, and clinical findings. Genetic findings were obtained in 107 neonates (42.6%), of which 67.3% (72/107) had pathogenic/likely pathogenic/variants of uncertain significance (P/LP/VUS) gene variations and 32.7% (35/107) had P/LP/VUS copy number variations (CNVs). The diagnostic rates of clinical exome sequencing were similar to those of exome sequencing. The logistic regression model revealed that CAD neonates with craniofacial abnormalities (OR = 4.15, 95% CI 2.29-7.53) or cardiovascular malformation (OR = 2.09, 95% CI 1.14-3.84) are more likely to be attributed to genetic causes. Follow-up analysis revealed that, compared to those in the undiagnosed group, the number of neonates whose care was withdrawn or who died was higher in the genetically diagnosed group (P < 0.05). This study identified a high incidence of genetic causes in critically ill neonates with CADs, with a combination of single-nucleotide variations and CNVs among the genetic causes of CAD. These findings highlight potential of NGS in the genetic testing of critically ill neonates with CADs.

Clinical features and treatment outcomes of Castleman disease in children: a retrospective cohort in China.

Castleman disease (CD) is a rare lymphoproliferative disorder of undetermined etiology. Unicentric CD (UCD) and multicentric CD (MCD) are two phenotypes of CD diagnosed by the histopathology of lymph nodes. We attempted to describe a pediatric CD cohort to optimize the management of this disease. We reviewed the medical records of pediatric patients diagnosed with CD between April, 2004, and October, 2022, at the Children's Hospital of Fudan University. Prognosis information was collected in January, 2023, by telephone inquiry. Twenty-two patients with UCD and 2 patients with MCD were identified, all with hyaline vascular (HV) type. The median ages at diagnosis were 10.75 years (IQR 8, 12.81) for UCD and 14.42 years (IQR 13.42, 15.42) for MCD. The most common lesion location of UCD was the neck (9/22, 40.91%) and abdomen (9/22, 40.91%). Systematic symptoms occurred on 10/22 (45.45%) patients with UCD and 1/2 (50%) patients with MCD, and abnormal laboratory indexes were detected in both. Resection and biopsy were performed on all patients. One out of two patients with MCD also received rituximab for upfront therapy. After a median of 4 years (IQR 1.5, 6) of follow-up time, the overall survival was 100% and the complete remission rate in UCD was 63%. There was no relapse or progression. CONCLUSIONS: Our series demonstrated that HV-UCD was the most common type in children. Resection and biopsy were used for both deterministic diagnoses and treatments. Despite the high possibility to develop systematic inflammation, children with CD showed promising outcomes. WHAT IS KNOWN: • Castleman disease is a rare lymphoproliferative disorder with limited cohort studies, especially in pediatrics. • The ubiquity of delayed confirmations and misdiagnoses points to a lack of knowledge about etiology and characteristics, which is a prerequisite for novel therapeutics. WHAT IS NEW: • We retrospectively reviewed and analyzed the clinical and pathological symptoms, laboratory and imaging features, and treatment outcomes of a Chinese pediatric cohort with Castleman disease. • Our work may improve the recognition and optimize the management of this rare disease in children.

Application of 3-Dimensional White-Light Scanning to Observe the Lip and Nose Morphology of Chinese Children.

OBJECTIVE: To establish a database and study the growth and development of Chinese children's nasolabial units by 3-dimensional (3D) white-light scanning technology. MATERIALS AND METHODS: From January 2018 to January 2019, 528 patients were selected preoperatively in our hospital, without cranial or maxillofacial deformities. The patients were placed in supine positions in a state of sedation, and the authors used a 3D white-light scanner to scan the children's faces to measure the 3D morphological structure of their nasolabial areas. The results were presented with Graphpad Prism 8.0.2 to explore correlations and analyze trends. RESULTS: The selected Chinese children consisted of 356 males (67.4%) and 172 females (32.6%). All nasolabial subunits grew fastest at 0 to 2 years old and at a slower rate from 2 to 15 years old, whereas the relevant growth of different sex was similar among contemporaries. CONCLUSIONS: Three-dimensional white-light scanning can quickly and accurately gain 3D imaging of nasolabial soft tissue to build a normal morphology database. Furthermore, every nasolabial subunit grows fastest at the age of 0 to 2 years.

Partial Glossectomy Combined With Radiofrequency Ablation for Macroglossia in Beckwith-Wiedemann Syndrome.

OBJECTIVE: As the most common feature of Beckwith-Wiedemann syndrome (BWS), macroglossia may influence the quality of life, maxillofacial growth, and speech development of children. The retrospective study aimed to investigate the therapeutic effect of partial glossectomy combined with radiofrequency ablation (RFA) for macroglossia patients in BWS. METHODS: A retrospective study was conducted in BWS-derived macroglossia patients who underwent partial glossectomy combined with RFA from May 2019 to January 2021. In total, 35 patients consisting of 17 males and 18 females met the inclusion criteria and underwent surgery by the same plastic surgeon. Demographic characteristics, BWS features, operation details, preoperative and postoperative outcomes, satisfaction evaluations, and subgroup analysis were collected and assessed. RESULTS: Of the 35 patients involved, the average age at the time of surgery was 14.05±8.08 months, and the average surgery duration was 48.17±6.72 minutes. Only 1 patient suffered ventral tongue wound dehiscence, and the rest of the patients did not develop any other complications. The severity and frequency of tongue protrusion, drooling, snoring, and feeding difficulty were significantly ameliorated. The patient's parents showed satisfaction towards the overall surgery, tongue's appearance, and tongue's motor function. Tongue's height decreased from 32.09±1.16 mm before the operation to 29.29±1.33 mm after the operation. CONCLUSION: The partial glossectomy combined RFA exerts a safe, effective and viable technique to treat BWS-derived macroglossia.

Case report: A novel truncating variant of BCL11B associated with rare feature of craniosynostosis and global developmental delay.

Craniosynostosis is a premature fusion of cranial sutures, resulting in abnormally shaped skull and brain development disorder. The description of craniosynostosis in patients with BCL11B mutations is rare. Here, we firstly report a 25-month-old Chinese boy with a novel frameshift variant in BCL11B gene. The patient was identified c.2346_2361del by whole-exome sequencing and was confirmed to be de novo by parental Sanger sequencing. This patient presented clinical phenotype of craniosynostosis as well as global developmental delay. He had a small mouth, thin upper lip, arched eyebrows, a long philtrum, midfacial hypoplasia and craniosynostosis. Brain MRI showed brain extracerebral interval and myelination changes, and brain CT with 3D reconstruction showed multi-craniosynostosis. Our study expands the clinical phenotypes of patients with BCL11B gene mutation, and our findings may help guide clinical treatment and family genetic counseling.

Comparing the Increased Intracranial Volume From Different Surgical Methods for Syndromic Craniosynostosis.

PURPOSE: Fronto-orbital advancement (FOA) is the traditional surgical method used to increase intracranial volume, but posterior cranial vault distraction osteogenesis (PVDO) has been gaining popularity as an initial treatment option. This study aimed to compare the effects of FOA and PVDO on intracranial volume. PATIENTS AND METHODS: Sixteen patients with multiple-suture synostosis and severe intracranial volume restriction were treated with FOA or PVDO at Children's Hospital of Fudan University between January 2016 and December 2019. Data on age at surgery, sex, preoperative intracranial volume, and postoperative intracranial volume were collected. RESULTS: Seven patients underwent FOA and 9 underwent PVDO. All patients underwent surgery for the first time, and the surgeries were performed by the same physician. There was no statistically significant difference in age at surgery or in the intracranial volumes before and after surgery between the 2 groups ( P >0.05). There was a statistically significant difference in the intracranial volume changes between the 2 groups before and after surgery ( P =0.028). CONCLUSIONS: Posterior cranial vault distraction osteogenesis provided statistically greater intracranial volume expansion than FOA.

The Cellular and Molecular Landscape of Synchronous Pediatric Sialoblastoma and Hepatoblastoma.

Sialoblastoma (SBL) is an infrequent embryonal malignant tumor originating from the salivary gland, resembling primitive salivary gland anlage, whereas hepatoblastoma (HB) is the most common pediatric liver malignancy. The simultaneous occurrence of both tumors is extremely rare. Here we reported a case of a 6-month-old infant diagnosed with synchronous SBL and HB. The patient received neoadjuvant chemotherapy followed by surgical resection. Fresh tissues of both tumors were collected before and after chemotherapy, which were further profiled by whole exome sequencing (WES) and single-cell RNA sequencing (scRNA-seq). WES analysis revealed potential somatic driver mutation PIK3CA p.Glu454Lys for SBL and canonical mutation CTNNB1 p.Ser45Pro for HB. No shared somatic variants or common copy number alterations were found between SBL and HB primary tumor samples. Though scRNA-seq, single-cell atlases were constructed for both tumors. SBL may recapitulate a pre-acinar stage in the development of salivary gland, including basaloid, duct-like, myoepithelial-like, and cycling phenotypes. In the meantime, HB was composed of tumor cells resembling different stages of the liver, including hepatocyte-like, hepatic progenitor-like, and hepatoblast-like cells. After chemotherapy, both tumors were induced into a more mature phenotype. In terms of transcriptional signatures, SBL and HB showed enhanced expression of epithelial markers KRT8, KRT18, and essential embryo development genes SDC1, MDK, indicating the disruption of normal embryo epithelium development. Finally, heterozygous deleterious germline mutation BLM and FANCI were identified which could predispose the patient to higher cancer risk. It partially explained the reason for the co-occurrence of SBL and HB. Taken together, we provided valuable resources for deciphering cellular heterogeneity and adaptive change of tumor cells after chemotherapy for synchronous SBL and HB, providing insights into the mechanisms leading to synchronous pediatric tumors.

Dysregulation of the HOTAIR-miR-152-CAMKIIα Axis in Craniosynostosis Results in Impaired Osteoclast Differentiation.

Craniosynostosis is one of the most common craniofacial deformities demanding surgical treatment in infancy. LncRNA HOTAIR has verified its important role in osteogenesis and osteoarthritis. However, whether HOTAIR plays an essential role in the development of craniosynostosis is still unclear. In this study, we aimed to investigate the molecular role of HOTAIR in the osteoclast function and development of craniosynostosis.For osteoclast differentiation, RAW264.7 cells were induced by 50 ng/ml of RANKL and 10 ng/mL M-CSF, followed by TRAP staining. Cell proliferation and apoptosis were assayed by the CCK-8 kit and Annexin V-FITC apoptosis detection kit, respectively. The expression of HOTAIR was determined in PBMCs by qRT-PCR. Protein levels of all those involved genes were measured by Western blot assay. A luciferase reporter assay was used to determine the miRNA target validation. The HOTAIR expression in PBMCs from children with craniosynostosis was significantly downregulated. The results of cell proliferation and apoptosis assays indicated that silencing of HOTAIR could inhibit osteoclast differentiation and increase cell apoptosis. Moreover, the luciferase reporter assay revealed that the regulatory axis and HOTAIR-miR-152-CAMKIIα were the regulatory mechanisms of HOTAIR in the osteoclast function and development of craniosynostosis.In this study, our data showed that HOTAIR could promote osteoclast differentiation by binding miR-152. Furthermore, the HOTAIR/HOTAIR-miR-152-CAMKIIα axis was found to regulate osteoclast differentiation. These results indicate that the HOTAIR plays a crucial role in the development of osteoclasts.

Propranolol inhibits the angiogenic capacity of hemangioma endothelia via blocking ß-adrenoceptor in mast cell.

BACKGROUND: Propranolol, a non-selective blocker of the ß-adrenoceptor (AR), is a first-line treatment for infantile hemangioma (IH). Mast cells have been implicated in the pathophysiology of propranolol-treated hemangioma. However, the function of mast cells remains unclear. METHODS: HMC-1s (Human mast cell line) having been treated with propranolol for 24 h were centrifuged, washed with PBS twice, and maintained in cell culture medium for another 24 h. The supernatants with propranolol which were named as propranolol-treated HMC-1s supernatants were obtained. The expression of cytokines and mediators was examined among HMC-1s dealt with propranolol. HemECs (hemangioma endothelial cells) were co-cultured with propranolol-treated HMC-1s supernatants, and their proliferation and apoptosis were investigated. The autophagic-related protein was examined in HemECs using immunoblot. RESULTS: In propranolol-treated HMC-1s, the expressions of ADRB1 (ß1-AR) and ADRB2 (ß2-AR) were reduced by 70% and 60%, respectively, and that of cytokines and mediators were reduced. The proliferation was decreased, but apoptosis and autophagy were induced in HemECs treated with propranolol-treated HMC-1s supernatants. However, propranolol can work well in shRNA-ADRB1 or shRNA-ADRB2 transfected HMC-1s. CONCLUSIONS: Propranolol inhibit the proliferation of HemECs and promote their apoptosis and autophagy through acting on both ß1 and ß2 adrenoceptor in mast cell. IMPACT: Treated with propranolol, ß1, and ß2 adrenoceptor on human mast cell expression was reduced significantly. After hemangioma endothelial cell treated with the supernatants from propranolol-treated human mast cell, its proliferation was decreased, but apoptosis and autophagy were significantly induced. Propranolol can work well in shRNA-ADRB1 or shRNA-ADRB2 transfected HMC-1s. Mast cells may have a role in the action of propranolol in infantile hemangioma through both ß1 and ß2 adrenoceptors to inhibit the angiogenic capacity of hemangioma endothelial cells.

Phenotypes and epigenetic errors in patients with Beckwith-Wiedemann syndrome in China.

BACKGROUND: Beckwith-Wiedemann syndrome (BWS) is primarily caused by epigenetic errors. This study aimed to analyze the relationship between the epigenetic errors and phenotypes of BWS and to evaluate the efficacy of diagnosing BWS using patients' clinical characteristics. METHODS: Patients clinically diagnosed with BWS were subjected to methylation-specific multiplex ligation-dependent probe amplification (MS-MLPA) for (epi)genotyping. The patients' clinical characteristics were analyzed and compared using regression models. The diagnostic efficacy of previous criteria and scoring systems was compared using area under the receiving operating curve (ROC). RESULTS: The most common clinical features observed in BWS patients were macroglossia (83.2%), abdominal wall defects (71.3%), and ear creases/pits (55.3%). Patients with the loss of methylation at imprinting control 2 (IC2-LOM) and gaining of methylation at imprinting control 1 (IC1-GOM) subtypes had significantly higher frequencies of ear creases/pits and facial nevus flammeus, and visceromegaly, respectively. Paternal uniparental isodisomy (pUPD) was characterized by significantly less macroglossia but more hemihypertrophy. The area under the curve (AUC) was comparably good in both recently developed scoring systems (0.87 for Ibrahim and 0.82 for Brioude.) and in the scoring system developed using the current cohort (0.88). CONCLUSIONS: This study, which is the largest cohort study of BWS cases in China published to date, confirmed the diagnostic efficacy of a recently developed symptom-based BWS scoring system in a Chinese population. Significant differences exist between the phenotypes of BWS epigenetic subtypes; however, the pattern is similar between Asian and European populations.

Application of Opposing Triangle Flaps in Three Different Kinds of Conjoined Twins.

BACKGROUND: Conjoined twins are a rare congenital anomaly. If separation of the conjoined organs is feasible, reconstruction of the skin and tissue defects is a challenge for the plastic surgeon. This article describes the use of opposing triangle flaps in the separation of three different kinds of conjoined twins. METHODS: Plastic surgeons measured each conjoined area and designated the vertical length as a and the width as b. The length of the base of the opposing triangle flap was calculated to match a, and the height of the triangle to match b. RESULTS: After detailed calculations and careful surgery, the area of the opposing triangle flaps nearly covered the areas exposed after separation, and the three conjoined twins achieved primary closure of their wounds. The pygopagus and ischiopagus twins recovered uneventfully. The omphalopagus twins developed a wound infection, but after daily wound care, the twins recovered within a week. CONCLUSIONS: With precise calculations, the opposing triangle flap is a feasible and effective method for defect closure after separation of conjoined twins in certain cases. Clinicians may prefer this technique because it avoids the complications and second surgery necessitated by tissue expanders.

Separation of pygopagus, omphalopagus, and ischiopagus with the aid of three-dimensional models.

BACKGROUND: The three-dimensional (3D) technique provides with accurate anatomical information. We present the separation surgeries for three different kinds of conjoined twins with the aid of three-dimensional techniques. METHOD: For the pygopagus twins, a pelvic and lower vertebral model was made. For the omphalopagus and ischiopagus, their enhanced computed tomography (CT) scan images were transferred to the Computer-Assisted Surgery Planning System (CASP) (Hisense, Qingdao, China) to generate the 3D models. RESULT: In the case of the pygopagus twins, the 3D model clearly showed that their coccyges were joined at a 120°angle from each other horizontally which suggested that the blind-end orifice on their back was a pilonidal sinus, which separated the normal sphincter into two halves. In the omphalopagus, the 3D model revealed one of the branches of each twin's hepatic vein was connected with the other's. The 3D model of the ischiopagus twins revealed that both of the twins had duplicated bladders and each baby's duplicated bladders united with one of the bladders of the other baby and a single rectum passing through the middle of the four bladders. CONCLUSION: 3D techniques could provide more detailed anatomical information, which is helpful in planning procedures for such complicated separation surgery. LEVELS OF EVIDENCE: Level IV.

Treatment of Children With Congenital Severe Blepharoptosis by Frontalis Aponeurosis Flap Advancement Under General Anesthesia in a Single Incision.

PURPOSE: This study aimed to investigate the effect of frontalis aponeurosis flap advancement in children with congenital severe blepharoptosis. METHODS: A total of 23 cases (25 eyes) of children who had congenital severe blepharoptosis and poor levator function (≤4 mm) and received frontalis aponeurosis flap advancement treatment in the Plastic Surgery Department of the Children's Hospital of Fudan University from January 1, 2013, to January 1, 2015, were retrospectively analyzed to evaluate the postoperative effects. RESULTS: All patients (age range, 6-27 months) were followed up for an average duration of 15.3 months. Twenty eyes (80%) had excellent effects, 2 eyes (8%) had good effects, and 3 eyes (12%) had poor effects. The average preoperative marginal reflex distance was 0.2 mm (-2 to 2 mm), and the postoperative average marginal reflex distance was 3.1 mm (1-4 mm). None of the patients showed hematoma, infection, keratohelcosis, symblepharon separation, ectropion, trichiasis, or other postoperative complications. CONCLUSION: Frontalis aponeurosis flap advancement could be used to treat congenital severe blepharoptosis with good short-term effects.

[Management of syndromic craniosynostosis using posterior cranial vault distraction osteogenesis:preliminary findings].

Objective: To explore the effect of managing syndromic craniosynostosis using posterior vault distraction osteogenesis. Methods: The authors conducted a retrospective cohort study of four children with syndromic craniosynostosis treated between 2015 January to 2016 March using posterior vault distraction osteogenesis. The posterior craniotomy was performed from vertex, biparietally to a point above the occipital protuberance. Two distraction devices were fixed in the parasagittal,collinear position. After a latency of 3 days, the device was activated at 0.5 mm/day. After the distraction, the consolidation period was about 6 months. Results: The average distraction distance was 27.3 mm (range,25 to 30 mm).Cerebrospinal fluid leak happened in one patient. After taken the 3D CT scan, all of them were undertaken the second operation of removing the distraction devices. All the patients were followed up at a mean of 12.8 months (range,7 to 20 months). Conclusions: It is effective to enlarge the posterior cranial vault using distraction osteogenesis for the syndromic craniosynostosis.

Immunohistochemical analysis of orbicularis oris muscle fiber distribution at the philtrum in healthy infants.

OBJECTIVE: To characterize the fiber-type distribution of the orbicularis oris muscle at the philtrum in healthy infants by immunohistochemistry and examine the relationship between orbicularis oris and philtrum structure. METHODS: Samples of the upper lip were obtained from two infant cadavers. Serial sagittal sections were obtained at the midline of the philtral dimple, unilateral philtral ridge, and the lateral side. Three sections from each site were prepared for immunohistochemical staining using myosin heavy chain fast fiber (MHCf) and myosin heavy chain slow fiber (MHCs) antibodies to determine the ratio of fast to slow skeletal muscle fibers. RESULTS: The ratio of fast to slow muscle fibers differed significantly among the superficial orbicularis oris muscle (98.30%:1.13%), deep pars peripheralis (95.30%:3.14%), and deep pars marginalis (91.31%:5.74%), with a significantly higher percentage of slow fibers in the pars marginalis compared to pars peripheralis (P=0.002) and fast fibers in the superficial muscle compared to pars marginalis and peripheralis (both P=0.000). Similarly, the fast:slow fiber ratio differed among the superficial philtral dimple (95.88%:2.41%), superficial philtral ridge (98.52%:1.11%), and superficial midlateral philtral ridge (99.07%:0.66%), with a higher percentage of fast fibers higher on the lateral side of the superficial philtral ridge than at the philtral ridge (P=0.030) and higher at the philtral ridge than the philtral dimple (P=0.001). The fast:slow fiber ratio did not differ within the pars peripheralis at the philtral dimple (93.94%:4.19%), philtral ridge (94.49%:3.84%), and lateral philtral ridge (95.79%:2.70%) (all P>0.05). CONCLUSIONS: Philtum structure is likely determined in part by the distribution of muscle fiber types among philtral dimple, ridge, and lateral side. These differences should be considered in cleft lip repair.

Mandible traction with wires for the treatment of upper airway obstruction caused by Pierre Robin sequence in Chinese infants: preliminary findings.

Pierre Robin sequence (PRS) is a congenital abnormality that may cause upper airway obstruction requiring surgical intervention. This preliminary study aimed to examine the feasibility and effectiveness of mandible traction with wires for the treatment of upper airway obstruction caused by PRS in Chinese infants. Measures of interest included transcutaneous oxygen saturation before and after surgery, duration of surgery and traction, complications, and CT findings. Seven infants were included in the study (mean birth weight: 2485 g, range: 2405-2570 g); four were born preterm and three were born full term. Mean age at surgery was 13.7 days (range: 2-28 days), mean duration of surgery was 16.6 min (range: 13-25 min) and mean duration of traction was 26.6 days (range: 21-35 days). Mean follow-up was 6.2 months (range: 1-11 months). No infant experienced severe complications. All infants experienced increases in transcutaneous oxygen saturation after surgery. Mean transcutaneous oxygen saturation was 82% before surgery and 98% after surgery. Follow-up morphology of the mandible was excellent. There was no upper airway obstruction, and short-term growth and development were satisfactory. These preliminary findings suggest that mandibular traction with wires may be an effective treatment for upper airway obstruction caused by PRS in Chinese infants.